摘要目的：探讨真皮神经鞘黏液瘤（ dermal nerve sheath myxoma，DNSM）和Neurothekeoma （ NTK）的临床病理学特征和鉴别诊断。方法对比性分析9例DNSM和8例NTK的临床资料、病理学形态和免疫学表型，并复习相关文献。结果9例DNSM中，发生于手／手指3例，外耳2例，面部、背部、腹壁和腰部各1例。8例NTK中，发生于前臂2例，鼻翼、唇、肩部、锁骨上、小腿和手各1例。临床上均表现为皮肤缓慢性生长的结节或斑块，多为无痛性。大体上DNSM质地相对柔软，而NTK质地多较坚实。镜下显示DNSM和NTK均位于真皮和／或皮下，其中DNSM由多个清晰的小叶组成，小叶间及周边为纤维性间隔，NTK在镜下也常显示小叶状结构，但相对不清晰，除小叶外，还可见束状或旋涡状排列结构。 DNSM小叶内的瘤细胞密度低，间质富于大量的黏液，瘤细胞由梭形、星状或上皮样瘤细胞组成，呈交织的条索样排列，常可见合体样细胞。 NTK小叶内的瘤细胞密度相对较高，瘤细胞主要由上皮样或单核组织细胞样细胞组成，其中2例可见少量多核性巨细胞。8例NTK中5例为富于细胞型，2例为混合型，1例为黏液型。免疫组织化学标记显示，DNSM中的瘤细胞弥漫性表达S-100蛋白、胶质纤维酸性蛋白和SOX10；NTK中的瘤细胞主要表达CD10、CD68和微小眼转录因子，不表达 S-100蛋白和 SOX10。随访显示，各有1例 DNSM 和 NTK 局部切除术后复发。结论DNSM和NTK在临床和病理学上有一定的相似之处，但属于不同的肿瘤类型，前者为周围神经肿瘤，后者与纤维组织细胞性肿瘤相近。熟悉DNSM和NTK的病理学形态特点和免疫学表型有助于区分两者，并与其他具有相似形态的皮肤肿瘤相鉴别。

abstractsObjective To investigate the clinicopathologic characteristics and differential diagnosis of dermal nerve sheath myxoma ( DNSM) and neurothekeoma ( NTK).Methods Clinical, pathological features and immunohistochemical profiles in 9 cases of DNSM and 8 cases of NTK were comparatively studied.The literature was reviewed.Results The involved site of 9 DNSMs included the hand/fingers (n=3), ear (n=2), face, back, abdominal wall and waist (1 case each).Two of 8 NTKs arose in the forearm ( n=2 ) , one each in the nose, lip, shoulder, supraclavicular region, leg and hand.The most common presentation was a painless cutaneous nodule or plaque which grew slowly.Grossly, DNSM was often gelatinous, whereas NTK appeared relatively solid.Microscopically, both tumors were located in the dermis and/or subcutis.DNSM was composed of well-defined multiple lobules separated by fibrous septa.NTK also exhibited lobular or multinodular architecture, but was relatively ill-defined.Besides, fascicular or whorl-like arrangement was present in 3 cases of NTK.The lobules in DNSM consisted of a paucicellular proliferation of spindled, stellate and epithelioid cells forming interconnecting cords within abundant myxoid matrix.Small syncytial-like aggregates were readily noted.The constituted neoplastic cells in NTK were composed of ovoid to round epithelioid or histiocytoid cells.Scattered multinucleated giant cells were present in 2 cases.Based on the amount of myxoid matrix, 8 NTKs were further classified into cellular (5 cases), mixed (2 cases) and myxoid (1 case).By immunohistochemistry, neoplastic cells in DNSM were diffusely positive for S-100 protein, CD68, glial fibrillary acidic protein and SOX10, whereas neoplastic cells in NTK consistently expressed CD10 and microphthalmia transcription factor, with negativity for S-100 protein and SOX10.One patient each with DNSM and NTK experienced local recurrence due to incomplete excision. Conclusions Although DNSM and NTK share clinical and pathological features, they belong to different entities.Whereas the former is consistent with a peripheral nerve sheath tumor, the latter is more akin to fibrous histiocytic tumor. Familiarity with their cliniopathologic characteristics and distinctive immunophenotypes will help distinguishing these two entities, as well as in the differential diagnosis of cutaneous neoplasms with similar features.